RESUMO
The authors report the case of a 6 year old girl with bladder duplication, urethral duplication, genital system duplication associated with colonic duplication and low double anorectal anomalies. This patient presented two hemivertebrae at T9 and T11. This girl died a few days after admission from internal obstruction and septicemia. The embryological features especially the possibility of associating two different embryopathogenic mechanisms in the pathogenesis of this combined malformation (Split notochord syndrome and fissure of the urogenital system), and diagnostic and therapeutic aspects are studied in relation to this case and a review of the literature.
Assuntos
Anormalidades Múltiplas , Colo/anormalidades , Reto/anormalidades , Uretra/anormalidades , Bexiga Urinária/anormalidades , Vulva/anormalidades , Criança , Feminino , HumanosAssuntos
Úlcera Péptica , Doença Aguda , Adolescente , Criança , Pré-Escolar , Doença Crônica , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Úlcera Péptica/diagnóstico , Úlcera Péptica/patologia , Úlcera Péptica/terapiaRESUMO
Three nephroblastomas, including one bilateral tumour, have been detected in three children belonging to the same family and born from a consanguinous marriage. The clinical and diagnostic particularities compared to non familial "sporadic" nephroblastomas are studied. The pathogenesis with the incidence of chromosomal anomalies, the diagnosis and the frequency of bilateral diseases along with the associated deformities and a non invasive therapeutic attitude are discussed.